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Abstract Details

A Subacute Neurologic Presentation of Fibrosing Inflammatory Pseudotumor
Autoimmune Neurology
P10 - Poster Session 10 (8:00 AM-9:00 AM)
9-006
FIP is a tumor-like mass with a poorly understood pathogenesis. Characterized by fibrotic ground tissue and polyclonal mononuclear infiltrate, it may cause compression of surrounding organs. Intracranial FIP is rare but can present as pachymeningitis.
We describe a case of a patient with fibrosing inflammatory pseudotumor (FIP) who presented with headaches, vision, and hearing loss.
N/A

A 33-year-old African American male with a history of type 2 diabetes mellitus and hypertension presented with a severe frontal headache, progressively worsening hearing loss, and decreased left eye visual acuity. He had been recently admitted for an episode of right eye vision loss with incomplete recovery. An MRI of the brain showed diffuse pachymeningitis with infiltration of the right orbital apex, bilateral trigeminal nerves, right internal carotid artery, and internal auditory meatus. A right pharyngeal mass was also found, which showed a dense fibrotic process on biopsy. A diagnosis of hypertrophic pachymeningitis was rendered, which led to consideration of a broad differential of subacute infectious processes such as tuberculous meningitis, inflammatory etiologies including neurosarcoidosis, and granulomatous polyangiitis, and malignancy. Cerebrospinal fluid (CSF) studies showed mild lymphocytic pleocytosis, normal protei with evidence of intrathecal antibody synthesis. Based on the workup, a diagnosis of FIP was made. He was treated with high-dose intravenous steroids. He improved clinically, which was further substantiated on follow-up imaging. As such, he was discharged on oral steroids.

FIP is a mixed inflammatory-fibrosing disease, which results in compression of surrounding organs. Widely described in the rheumatological literature, this entity is now gaining recognition in clinical neurology. As such, it should be recognized in the differential diagnosis of multiple cranial neuropathies.

Authors/Disclosures
Mattia Rosso, MD (Medical University of South Carolina)
PRESENTER
Dr. Rosso has nothing to disclose.
Cori A. Cummings, MD (MUSC) Dr. Cummings has nothing to disclose.
Mini Singh, MD Dr. Singh has nothing to disclose.